Necrotizing lymphadenitis: A review of clinicopathological, immunohistochemical and ultrastructural studies

Abstract

Necrotizing lymphadenitis (NEL) has been reported to be a reactive process described under differing terminology by Fujimoto et al. (1972), Kikuchi (1972), Wakasa et al. (1973) and other Japanese pathologists. Recently, this type of lymphadenitis has also been reported in America and Europe. In Japan, NEL is observed more frequently in the northern area, however, no characteristic seasonal occurrence has been noted. The disease affects young females more than males, particularly from the third and fourth decades onwards. Common cold-like symptoms, lymphadenopathy of the cervical region and leukopenia are characteristic clinical findings in the early stages. Morphological features of the involved lymph nodes include the presence of numerous immunoblasts, histiocytes and macrophages, the latter with phagocytized nuclear debris derived from degenerated lymphocytes. However, granulocytes are generally absent. Tubular inclusions are observed ultrastructurally. Immunohistochemical studies of peripheral blood using monoclonal antibodies have revealed that the helper/suppressor (Leu 3a/2a) ratio increases gradually with the clinical course because of a decrease in Leu 2a + cells. The pathogenesis of NEL is uncertain, but it has been speculated that there is cytolytic infection of lymphocytes by a virus or other organism, accompanied by secondary blastic transformation of suppressor T-lymphocytes.

Kikuchi-Fujimoto Disease With Prolonged Fever in Children

“…[11][12][13][14]16 KFD is more prevalent in Asians, particularly in Far East nations including Korea. [5][6][7][8][9][10] Tanaka et al 9 reported that 2 HLA class II Cervical lymphadenopathy and fever are the most common presentations of the disorder. However, the clinical presentation may vary from patient to patient.…”

“…Although lymphadenopathy usually affects the cervical lymph nodes, the axillary lymph nodes and lymph nodes in other regions can be affected. [3][4][5][6][7][8][9][10]14,19 The size of the affected lymph nodes usually are 2 to 3 cm, but sometimes these nodes can be Ͼ5 cm. An affected lymph node is solid, movable, and painful but not suppurative.…”

“…This disease has now been recognized in many other countries and has also been referred to as Kikuchi's disease, histiocytic necrotizing lymphadenitis, focal histiocytic lymphadenitis, and subacute necrotizing lymphadenitis. [3][4][5][6][7][8][9][10] The characteristic clinical presentation of the disorder includes cervical lymphadenopathy and prolonged fever. Its natural course is usually benign, and the clinical symptoms and signs disappear within a few months without specific treatment.…”

Kikuchi-Fujimoto disease: case report and review

“…Most patients with Kikuchi disease have a normal blood count [8], even though leukopenia is observed in 20-32% [9,21]. Atypical lymphocytes are reported in up to 25% of patients [18].…”

SLE Developing in a Follow-Up Patient of Kikuchi’s Disease: A Rare Disorder

“…One ultrastructural study proposed that Kikuchi's syndrome reflects a self-limited, SLE-like autoimmune condition which is caused by virus-infected transformed lymphocytes [8]. In reviews, some patients who were initially diagnosed with Kikuchi's disease were reported to have subsequently developed systemic lupus erythematosus, and an ANA test should be performed in patients with suspected Kikuchi's syndrome in order to exclude this diagnosis [9,10]. Examination of the lymph node histopathology, in correlation with the clinical features, helps in differentiating SLE from KFD.…”